Inflammatory myopathies: certain autoimmune conditions carry a significant risk of pulmonary complications

Clinicians from ten centres within the Assistance-Publique Hôpitaux de Paris (APHP) compiled and compared the clinical and laboratory data of 154 adults with positive anti-Ku autoantibodies over the period 2010–2025:

  • 60 (40% of the sample) developed interstitial lung disease (ILD) at some point during their course of the disease,
  • chest imaging was analysed against international criteria for assessing the presence and progression of ILD,
  • the median age was 56 years,
  • the progression of ILD, measured over time, was associated with reduced survival, particularly in men.

This research, based on a large cohort, will help to better understand the natural history of this rare disease.